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Patients with Down syndrome (DS) are at 10to 36-fold higher risk of developing leukemia (Roy et al., 2009). In children with DS aged 4 years or older, acute lymphoblastic leukemia (ALL) is the predominant type of leukemia just as it is in the general pediatric population, whereas acute myeloid leukemia (AML) is more common than ALL in patients with DS less than 4 years of age. Interestingly, acute megakaryoblastic leukemia (AMKL), a rare subtype of AML in non-DS patients, comprises 62-86% of AML cases in children with DS (Hitzler, 2007; Roy et al., 2009), which will be referred to as AMKL-DS hereafter. Furthermore, hematological abnormalities that are indistinguishable from AMKL-DS occur in about 10% of neonates with DS but spontaneously disappear within several months of life. This disorder has been given a variety of names, including transient leukemia (TL), transient myeloproliferative disorder (TMD) and transient abnormal myelopoiesis (TAM). In 20-30% of patients with TL, AMKL-DS develops later through the stage of myelodysplastic syndrome (MDS) within 4 years. These disorders, namely, TL, MDS and AMKL-DS, in young children with DS have many unique features and had been considered a disease entity that was called “Myeloid leukemias of Down syndrome”, then later renamed “Myeloid proliferations related to Down syndrome” in the current World Health Organization (WHO) Classification published in 2008. This review summarizes recent data on clinical, cellular and molecular biological aspects of these myeloid neoplasms with special reference to the origin of neoplastic cells, the organs where they arise and multistep model of leukemogenesis.

Unique Myeloid Leukemias in Young Children with Down Syndrome: Cell Origin, Association with Hematopoietic Microenvironment and Leukemogenesis